Examination of the Association of Sex and Race/Ethnicity with Appearance Concerns: A
Scleroderma Patient-centered Intervention Network (SPIN) Cohort Study
Lisa R. Jewett, MSc1,2; Linda Kwakkenbos, PhD1,3,4; Marie-Eve Carrier, MSc1; Vanessa L.
Malcarne, PhD5,6; Susan J. Bartlett, PhD7,8; Daniel E. Furst, MD9; Karen Gottesman, BA10;
Maureen D. Mayes, MD11; Shervin Assassi, MD11; Diana Harcourt, PhD12; Heidi Williamson,
DHealthPsy12; Sindhu R. Johnson, MD, PhD13; Annett Körner, PhD1,2; Virginia Steen, MD14;
Rina S. Fox, MS, MPH6; Shadi Gholizadeh, MS6; Sarah D. Mills, MS, MPH6; Jacqueline C.
Molnar1; Danielle B. Rice, MSc1,15; Brett D. Thombs, PhD1-3,8,15-17; and the SPIN Investigators18
1Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec,
Canada; 2Department of Educational and Counselling Psychology, McGill University, Montreal,
Quebec, Canada; 3Department of Psychiatry, McGill University, Montreal, Quebec, Canada;
4Behavioural Science Institute, Clinical Psychology, Radboud University, Nijmegen, the
Netherlands; 5Department of Psychology, San Diego State University, San Diego, California,
USA; 6San Diego State University/University of California, San Diego Joint Doctoral Program in
Clinical Psychology, San Diego, California, USA; 7McGill University Health Center, Montreal,
Quebec, Canada; 8Department of Medicine, McGill University, Montreal, Quebec, Canada;
9Division of Rheumatology, Geffen School of Medicine at the University of California, Los
Angeles, Los Angeles, USA; 10Scleroderma Foundation, USA; 11 Department of Internal
Medicine, Division of Rheumatology, University of Texas McGovern Medical School, Houston,
Texas, USA; 12Centre for Appearance Research, University of the West of England, Bristol,
United Kingdom; 13Toronto Scleroderma Program, Division of Rheumatology, Department of
1
Medicine, Toronto Western and Mount Sinai Hospitals, Toronto, Ontario, Canada; 14Department
of Medicine, Georgetown University, Washington, DC, USA; Departments of
15Psychology,16Epidemiology, Biostatistics and Occupational Health, and 17School of Nursing,
McGill University, Montreal, Quebec, Canada; 18SPIN Investigators: Murray Baron, McGill
University, Montréal, Québec, Canada; Frank van den Hoogen, Radboud University Medical
Center and Sint Maartenskliniek, Nijmegen, The Netherlands; Dinesh Khanna, University of
Michigan, Ann Arbor, Michigan, USA; Luc Mouthon, Université Paris Descartes, Paris, France;
Warren R. Nielson, St. Joseph’s Health Care, London, Ontario, Canada; Serge Poiraudeau,
Université Paris Descartes, Paris, France; Robert Riggs, Scleroderma Foundation, Danvers,
Massachusetts, USA; Maureen Sauve, Scleroderma Society of Ontario, Hamilton, Ontario;
Fredrick Wigley, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA;
Isabelle Boutron, Université Paris Descartes, and Assistance Publique-Hôpitaux de Paris, Paris,
France; Angela Costa Maia, University of Minho, Braga, Portugal; Ghassan El-Baalbaki,
Université du Québec à Montréal, Montréal, Québec, Canada; Carolyn Ells, McGill University,
Montréal, Québec, Canada; Cornelia van den Ende, Sint Maartenskliniek, Nijmegen, The
Netherlands; Kim Fligelstone, Scleroderma Society, London, UK; Catherine Fortune,
Scleroderma Society of Ontario, Hamilton, Ontario, Canada; Tracy Frech, University of Utah,
Salt Lake City, Utah, USA; Dominique Godard, Association des Sclérodermiques de France,
Sorel-Moussel, France; Daphna Harel, New York University, New York, New York, USA;
Marie Hudson, McGill University, Montréal, Québec, Canada; Ann Impens, Midwestern
University, Downers Grove, Illinois, USA; Yeona Jang, McGill University, Montréal, Québec,
Canada; Ann Tyrell Kennedy, Federation of European Scleroderma Associations, Dublin,
Ireland; Maggie Larche, McMaster University, Hamilton, Ontario, Canada; Catarina Leite,
2
University of Minho, Braga, Portugal; Carlo Marra, Memorial University, St. John’s,
Newfoundland, Canada; Karen Nielsen, Scleroderma Society of Ontario, Hamilton, Ontario,
Canada; Janet L. Poole, University of New Mexico, Albuquerque, New Mexico, USA; Janet
Pope, University of Western Ontario, London, Ontario, Canada; Alexandra Portales, Asociación
Española de Esclerodermia, Madrid, Spain; Tatiana Sofia Rodriguez Reyna, Instituto Nacional
de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico; Anne A. Schouffoer,
Leiden University Medical Center and Haga Teaching Hospital, the Hague, Leiden, The
Netherlands; Russell J. Steele, Jewish General Hospital and McGill University, Montréal,
Québec, Canada; Maria E. Suarez-Almazor, University of Texas MD Anderson Cancer Center,
Houston, Texas, USA; Joep Welling, NVLE Dutch patient organization for systemic
autoimmune diseases, Utrecht, The Netherlands; Durhane Wong-Rieger, Canadian Organization
for Rare Disorders, Toronto, Ontario, Canada; Alexandra Albert, Université Laval, Québec,
Québec, Canada; Guylaine Arsenault, Sherbrooke University, Sherbrooke, Québec, Canada;
Lyne Bissonnette, Sherbrooke University, Sherbrooke, Québec, Canada; Gilles Boire,
Sherbrooke University, Sherbrooke, Québec, Canada; Alessandra Bruns, Sherbrooke University,
Sherbrooke, Québec, Canada; Patricia Carreira, Servicio de Reumatologia del Hospital 12 de
Octubre, Madrid, Spain; Lorinda Chung, Stanford University, Stanford, California, USA; Pierre
Dagenais, Sherbrooke University, Sherbrooke, Québec, Canada; Christopher Denton, Royal Free
London Hospital, London, UK; Robyn Domsic, University of Pittsburgh, Pittsburgh,
Pennsylvania, USA; James V. Dunne, St. Paul's Hospital and University of British Columbia,
Vancouver, British Columbia, Canada; Paul Fortin, Université Laval, Québec, Québec, Canada;
Anna Gill, Royal Free London Hospital, London, UK; Jessica Gordon, Hospital for Special
Surgery, New York City, New York, USA; Genevieve Gyger, Jewish General Hospital and
3
McGill University, Montréal, Québec, Canada; Ariane L. Herrick, University of Manchester,
Salford Royal NHS Foundation Trust, Manchester, UK; Joanne Manning, Salford Royal NHS
Foundation Trust, Salford, UK; Monique Hinchcliff, Northwestern University, Chicago, Illinois,
USA; Alena Ikic, Université Laval, Québec, Québec, Canada; Niall Jones, University of Alberta,
Edmonton, Alberta, Canada; Artur Jose de B. Fernandes, Sherbrooke University, Sherbrooke,
Québec, Canada; Suzanne Kafaja, University of California, Los Angeles, California, USA;
Nader Khalidi, McMaster University, Hamilton, Ontario, Canada; Benjamin Korman,
Northwestern University, Chicago, Illinois, USA; Patrick Liang, Sherbrooke University,
Sherbrooke, Québec, Canada; Ariel Masetto, Sherbrooke University, Sherbrooke, Québec,
Canada; David Robinson, University of Manitoba, Winnipeg, Manitoba, Canada; Sophie Roux,
Sherbrooke University, Sherbrooke, Québec, Canada; Elena Schiopu, University of Michigan,
Ann Arbor, Michigan, USA; Doug Smith, University of Ottawa, Ottawa, Ontario, Canada;
Robert Spiera, Hospital for Special Surgery, New York, New York, USA; Evelyn Sutton,
Dalhousie University, Halifax, Nova Scotia, Canada; Carter Thorne, Southlake Regional Health
Centre, Newmarket, Ontario, Canada; John Varga, Northwestern University, Chicago, Illinois,
USA; Pearce Wilcox, St. Paul's Hospital and University of British Columbia, Vancouver, British
Columbia, Canada; Vanessa C. Delisle, Jewish General Hospital and McGill University,
Montréal, Québec, Canada; Claire Fedoruk, Jewish General Hospital, Montréal, Québec,
Canada; Brooke Levis, Jewish General Hospital and McGill University, Montréal, Québec,
Canada; Katherine Milette, Jewish General Hospital and McGill University, Montréal, Québec,
Canada; Mia R. Pepin, Jewish General Hospital, Montréal, Québec, Canada; Jennifer Persmann,
Université du Québec à Montréal, Montréal, Québec, Canada.
4
Address for Correspondence and Reprints: Brett D. Thombs, PhD; Jewish General Hospital;
4333 Cote Ste Catherine Road, Montréal, Québec, Canada, H3T 1E4; Telephone: (514) 340-
8222 ext. 5112; Fax: (514) 340-8124; Email: [email protected].
Short Running Title: Appearance Concerns in SSc
5
ABSTRACT
Objective: Appearance concerns are common in systemic sclerosis (SSc) and have been linked
to younger age and more severe disease. No study has examined their association with sex or
race/ethnicity.
Methods: SSc patients were sampled from the Scleroderma Patient-centered Intervention
Network Cohort. Presence of appearance concerns was assessed with a single item, and medical
and sociodemographic information were collected.
Results: Of 644 patients, appearance concerns were present in 72%, including 421 of 565
women (75%), 42 of 79 men (53%), 392 of 550 patients who identified as White (71%), 35 of 41
who identified as Black (85%), and 36 of 53 who identified as another race/ethnicity (68%). In
multivariate analysis, women had significantly greater odds of reporting appearance concerns
than men (odds ratio (OR)=2.97, 95% confidence interval (CI)=1.78-4.95, p<.001). Black
patients had significantly greater odds of appearance concerns than White patients in unadjusted
(OR=2.64, 95% CI=1.01-6.34, p=.030), but not multivariate analysis (OR=1.76, 95% CI=0.67-
4.60, p=.250). Compared to a general population sample, appearance concerns were substantially
more common in SSc, particularly for men across all age groups and for younger women. The
most commonly reported features of concern were related to the face and head, followed by the
hands and fingers; this did not differ by sex or race/ethnicity.
Conclusion: Appearance concerns were common in SSc. Women were substantially more likely
than men to have appearance concerns. Although non-significant in multivariate analysis, Black
patients were more likely to have concerns than White patients, likely due to more severe
changes in appearance.
6
Disfiguring appearance changes, including telangiectasias, hand contractures, skin
pigmentation changes, digital ulcers, and altered facial features, are common in systemic
sclerosis (SSc). These appearance changes often affect body parts that are highly visible and that
play a central role in social interactions, such as the face, mouth, and hands [1-3]. Treatments can
lessen the impact of some SSc symptoms but do not alleviate manifestations of irreversible tissue
damage that affect appearance.
Among individuals with visible differences due to medical illness or injury, there is a
well-established relationship between the extent and severity of appearance changes and
psychological outcomes [4, 5]. Consistent with this, in SSc, the presence, severity, and perceived
noticeability of appearance changes are associated with greater body image dissatisfaction and
social discomfort, as well as poorer overall psychosocial and psychological functioning [6-12].
The association of younger age and greater appearance concerns is also well-established, both
among people with visible differences and in the general population [4, 13, 14]. Among 2,100
randomly sampled members of the UK general population, 70% of women and 50% of men 30
years or younger reported at least one appearance concern compared to 33% of women and 21%
of men 61 years or older [14]. Younger patients with SSc have also been found to experience
greater social discomfort related to appearance than older patients [7].
No published studies have investigated the degree to which sex and race/ethnicity may be
associated with appearance concerns in SSc, likely due to the small numbers of men and non-
White patients in most study samples. Among other groups of people with visible differences
(e.g., skin conditions, burn scarring, limb disfigurement), however, women experience more
worry about their appearance and greater general distress, social anxiety, and social avoidance
than men [15-20]. Less is known about the association of race/ethnicity and appearance concerns
7
due to visible differences. One survey of 458 adults with a variety of visible disfigurements
found that people from non-White racial/ethnic backgrounds experienced significantly greater
worry about their appearance and heightened concern that their condition was noticeable to
others than White respondents [20]. This may have been because people with darker skin tones
are more vulnerable to visible changes in skin pigmentation and report greater psychosocial
impact compared to individuals with lighter skin tones [21]. In SSc, skin involvement and
pigmentation changes are more common among Black patients than White patients, whereas
White patients are more likely to have telangiectasias [22].
The objective of the present study was to examine the association of sex and race/ethnicity
with the presence of appearance concerns, controlling for factors that are known to influence
appearance concerns (e.g., age, disease severity) in a large, international cohort of SSc patients.
We also compared the percentage of patients with SSc who reported the presence of appearance
concerns to rates previously published from a UK general population sample, stratified by sex
and age groups [14].
METHODS
The sample consisted of patients enrolled in the Scleroderma Patient-centered
Intervention Network (SPIN) Cohort [23] who completed baseline study questionnaires from
April 2014 through August 2015. Patients were enrolled at 21 SPIN centers in Canada, the USA,
and the UK. To be eligible for the SPIN Cohort, patients must have a confirmed diagnosis of SSc
according to the 2013 American College of Rheumatology/European League Against
Rheumatism classification criteria [24], be ≥ 18 years of age, have the ability to give informed
consent, be fluent in English or French, and have access and the ability to respond to
questionnaires via the Internet. The SPIN sample is a convenience sample. Eligible patients are
8
invited by attending physicians or supervised nurse coordinators from SPIN centers to participate
in the SPIN Cohort, and written informed consent is obtained. The local SPIN physician or nurse
coordinator completes a medical data form that is submitted online to initiate patient registration.
After completion of online registration, an automated welcoming email is sent to participants
with instructions for activating their SPIN account and completing SPIN Cohort measures
online. SPIN Cohort patients complete outcome measures via the Internet upon enrollment and
subsequently every three months. Patients who had complete data at their baseline assessment for
all variables necessary for the planned multivariate analysis were included. The SPIN Cohort
study was approved by the Research Ethics Committee of the Jewish General Hospital,
Montréal, Canada and by the research ethics committees of each participating center.
Sociodemographic Characteristics
Patients enrolled in the SPIN Cohort provided sociodemographic data, including sex,
race/ethnicity, age, education level, marital status, and employment status. Response options for
race/ethnicity differed slightly for patients from Canada, the USA, and the UK, consistent with
how racial/ethnic status is typically characterized in each country. In the Canadian sample,
patients could identify as White, Black, Aboriginal, Asian, Latin American, or Arab. In the USA
sample, patients could identify as White (non-Hispanic), African American or Black, Hispanic or
Latino, Asian, American Indian/Alaska Native, Native Hawaiian/Other Pacific Islander, or
Mixed-race. In the UK sample, patients could identify as White, African, or Asian. In the present
study, racial/ethnic status was collapsed into three categories across Canadian, American, and
UK samples, consisting of White, Black, or Other. Across countries, responses indicating White
racial/ethnic status were combined to create one White race/ethnicity category; responses
indicating Black, African American or Black, or African were combined to create one Black
9
race/ethnicity category; and other responses were combined to create one Other race/ethnicity
category.
Disease-related and Medical Characteristics
SPIN physicians or nurse coordinators provided disease and medical information,
including time since onset of the first non-Raynaud’s symptom (disease duration); disease
subtype (limited or diffuse); and presence of telangiectasias, skin pigmentation changes, hand
contractures, skin thickening on the fingers of both hands, and body mass index (BMI). Limited
SSc was defined as skin involvement distal to the elbows and knees only, whereas diffuse SSc
was defined as skin involvement proximal to the elbows and knees, and/or the trunk [25].
Telangiectasias were defined as the visible dilation of superficial cutaneous blood vessels that
collapse upon pressure and fill slowly when pressure is released [24]. Skin pigmentation changes
included either hyper- or hypo-pigmentation of the skin. In the present study, telangiectasias and
skin pigmentation changes were coded as present on the body and face, present on the body only,
or none. Hand contractures, which entail limitations in the range of motion of a joint, secondary
to tightening around the joint, were measured for small joints on the hands (i.e., proximal
interphalangeal joints, metacarpals, and/or wrists) and categorized as None/Mild (0-25%
limitation in range of motion), Moderate (25-50%), or Severe (>50%). Skin thickening on the
fingers of both hands was defined as skin thickening or hardening extending proximal to the
metacarpophalangeal joints [22].
Presence of Appearance Concerns
The Derriford Appearance Scale (DAS-24) [26, 27] is a self-report measure of distress
related to problems with appearance. It assesses social anxiety and avoidance related to self-
consciousness due to appearance. The DAS-24 has an introductory item, not included in the
10
scoring of the measure, that asks, “Is there any aspect of your appearance (however small) that
concerns you at all?” (yes/no). This item was used as the primary outcome in the present study.
Published prevalence of appearance concerns using this question is available for the UK general
population [14], which we used for comparison. In addition, for patients who answer yes to the
appearance concern question, the DAS-24 includes a further inquiry, “The aspect of my
appearance about which I am most sensitive or self-conscious is ….” with space for open
response. s If patients reported more than one aspect of appearance concern, the first listed was
used.
Data Analysis
Descriptive statistics were calculated for all sociodemographic and disease/medical
variables, including means and standard deviations (SDs) for continuous variables. Chi-square
tests were used for categorical variables, and a one-way Analysis of Variance was used for
continuous variables to compare patients on sociodemographic and disease/medical
characteristics across sex and race/ethnicity categories. For illustrative purposes, Bonferroni-
corrected comparisons were done to assess statistical significance between pairs of race/ethnicity
groups for variables with statistically significant overall tests. To maintain the family-wise error
rate < .05, the Bonferroni-corrected α for each of the three subgroup comparisons for each
variable was .0167.
The associations of sociodemographic variables (sex, race/ethnicity, age, marital status,
education level), and disease/medical variables (telangiectasias, skin pigmentation changes, hand
contractures, skin thickening on fingers, disease subtype, BMI) with the presence of appearance
concerns were assessed using binary logistic regression. All variables included in the regression
analysis were selected a priori based on previous research indicating variables likely to relate to
11
appearance concerns in SSc. Discrimination and calibration of the multivariate model were
assessed with the c-index and Hosmer-Lemeshow goodness-of-fit test statistic, respectively [28].
The c-index is the percentage of comparisons where patients with appearance concerns had a
higher predicted probability of having appearance concerns than patients without appearance
concerns for all possible pairs where patients were discrepant on outcome variable status. The
Hosmer-Lemeshow goodness-of-fit statistic is a measure of the accuracy of the predicted number
of cases of appearance concerns compared to the number of patients who actually reported
appearance concerns across the spectrum of probabilities. A relatively large p value indicates a
reasonably good model fit [28]. All analyses were conducted using SPSS (Version 22), and
statistical tests were two-sided with α < .05.
The percentage of women and men with SSc who reported appearance concerns was
compared to levels of appearance concerns reported in a sample from the general UK population
[14]. The relative risk of reporting appearance concerns was calculated for women and men,
separately, stratified by age groups. Race/ethnicity data were not provided for the UK population
sample, thus comparisons for these groups separately could not be conducted.
RESULTS
In total, 757 SSc patients completed baseline assessments. There were 717 who answered
the binary appearance concern item, of whom 644 had data for all variables included in the
multivariate model and were included in analyses. Of these, 463 (72%) indicated that there was
an aspect of their appearance that caused them concern. Rates were similar among the 73 patients
excluded for missing data, with 57 (78%) indicating that there was an aspect of their appearance
that caused them concern.
12
Sociodemographic and disease/medical characteristics are displayed in Table 1. Mean
age in the total sample was 55.3 years (Standard Deviation (SD) = 12.1), and the majority of
patients were White (N = 550, 85%) and married or living as married (N = 471, 73%). Mean
time since onset of the first non-Raynaud’s symptom was 11.5 (SD = 8.7) years.
There were 565 (88%) women and 79 (12%) men included. There were no statistically
significant differences between women and men for any sociodemographic or disease/medical
variables (see Table 1). There were 41 (6%) Black patients and 53 (8%) patients who identified
as a member of another racial/ethnic group. As shown in Table 1, White patients were older and
more likely to be married than Black patients, but less likely to have skin thickening on the
fingers and to have diffuse SSc (statistically significant, p < .0167). There were also statistically
significant differences between White and Black patients in presence of telangiectasias and skin
pigmentation changes. White patients were older and less likely to have diffuse SSc than patients
from other racial/ethnic groups (statistically significant, p < .0167). There were also statistically
significant differences in skin pigmentation changes between White patients and those from
other racial/ethnic groups (see Table 1).
Among women, 75% (421 of 565) reported appearance concerns, compared to 53% (42
of 79) of men (p < .001). Across racial/ethnic groups, 85% (35 of 41) of Black patients reported
appearance concerns, compared to 71% (392 of 550) of White patients and 68% (36 of 53) from
other racial/ethnic groups (p = .122).
Of the 463 patients who indicated that there was an aspect of their appearance that
caused them concern, 444 listed the specific feature of greatest concern. As shown in Table 2,
the most common category was the face/neck/head/mouth (41%), followed by the hands/fingers
13
(27%). Results were similar for female and male patients and patients classified as White, Black,
or Other racial/ethnic status.
As shown in Table 3, on an unadjusted basis, the odds of appearance concerns were
greater for female patients compared to male patients (OR = 2.70, 95% Confidence Interval (CI)
= 1.73-4.22, p < .001), and for Black patients versus the reference group, White patients (OR =
2.64, 95% CI = 1.01-6.34, p = .030). Patients with another racial/ethnic status did not have
significantly different odds compared to White patients (OR = 0.92, 95% CI = 0.52-1.65, p
= .788). Other variables that were significantly associated with the presence of appearance
concerns on an unadjusted basis included younger age, diffuse disease subtype, presence of
moderate hand contractures, and presence of skin thickening on the fingers.
In the multivariate analysis, only sex, age, and presence of moderate hand contractures
were significantly associated with appearance concerns, after controlling for covariates of
race/ethnicity, BMI, education level, marital status, disease subtype, telangiectasias,
pigmentation changes, and skin thickening on the fingers. The odds of appearance concerns for
female patients were significantly greater than for male patients (OR = 2.97, 95% CI = 1.78-
4.95, p < .001). Older patients were also less likely to report appearance concerns than younger
patients (OR = 0.98 per year, 95% CI = 0.96-0.99, p = .007), equivalent to a reduction of 18% in
the OR for every 10-year increase in age. The odds of reporting appearance concerns did not
differ significantly for Black versus White patients (OR = 1.76, 95% CI = 0.67-4.60, p = .250).
The odds of reporting appearance concerns for patients with moderate hand contractures were
almost twice those of patients with no or only mild hand contractures (OR = 2.05, 95% CI =
1.17-3.60, p = .012) (see Table 3). Model fit for the 11 predictors included was less than ideal
14
based on the Hosmer-Lemeshow test (2(8, N = 644) = 10.65, p = .022), and the c-index statistic
was 0.69.
As shown in Table 4, rates of appearance concerns were higher, across all age groups, for
women and men with SSc, compared to women and men from the general UK population sample
(14). The relative risk of reporting appearance concerns among SSc patients compared to the
general population was greater for men than for women across age groups. For both women and
men, the relative risk was substantially higher among older patients.
DISCUSSION
The main findings of this study were that women with SSc were substantially more likely
than men to report appearance concerns, controlling for sociodemographic and disease/medical
variables. Black racial/ethnic group membership was significantly associated with appearance
concerns at the bivariate level; however, the association was not statistically significant after
accounting for the influences of other sociodemographic and disease/medical variables.
Consistent with findings from previous studies, older age was significantly associated with
reduced odds of appearance concerns. Among disease variables, moderate hand contractures
were significantly associated with greater odds of appearance concerns. Compared to data from a
general population sample, both women and men with SSc were more likely to report appearance
concerns, although the relative risk was higher for men with SSc compared to women. In
addition, for both women and men, the relative risk was substantially higher, compared to the
general population, among older patients than among younger patients. The most commonly
reported features of concern were related to the face and head, followed by the hands and
fingers; this did not differ by sex or race/ethnicity.
15
The finding that female SSc patients had greater odds of experiencing appearance concerns
is consistent with previous research on the relationship between sex and visible differences,
which has highlighted that women tend to experience greater distress, social anxiety, and
difficulty adjusting to disfiguring appearance changes than men [16-18]. Although 85% of Black
patients reported appearance concerns compared to 71% of White patients, race/ethnicity was not
independently associated with appearance concerns in the multivariate analysis. It is possible that
the small number of Black people in the study could explain the non-significant result. This
finding may also have occurred because Black patients tended to be younger, have diffuse
disease, and have greater pigmentation changes and skin thickening on the fingers compared to
White patients, as these are all factors associated with appearance concerns.
Although men with SSc were less likely to report appearance concerns than women with
the disease, men with SSc had a greater relative risk of appearance concerns compared to the
general population sample than women. In both SSc and the general population, women are more
likely than men to have appearance concerns, but the increase in appearance concerns is much
greater for men with SSc. A similar phenomenon can be seen with respect to age. For both
women and men in the general population, the proportion of people with appearance concerns
drops substantially in older groups, particularly for people aged 61 and older. The proportion
also diminishes across age groups for people with SSc, but much less dramatically, and in SSc,
older people are more likely to report appearance concerns compared to the general population,
where worries about appearance have mostly subsided among older adults.
The finding that the majority of patients across sexes and race/ethnicities listed the face
and head body regions as the primary feature that caused them concern, followed by the hands
and fingers, is consistent with previous research in SSc, which has reported that appearance
16
changes to visible and socially relevant body regions are common and of the greatest distress to
patients [2, 6, 7, 9, 12]. While the categories of appearance concern in the present study were
broad, results showed that the general types of appearance concerns reported by patients were
fairly consistent across sex and racial/ethnic groups.
The present study has limitations that should be considered in interpreting results. First, the
SPIN Cohort constitutes a convenience sample of SSc patients receiving treatment at SPIN
recruiting centers, and patients at these centers may differ from those in other settings. SSc
patients in the SPIN Cohort also complete questionnaires online, which may further limit the
generalizability of findings. An additional limitation relates to the nature of disease
characteristics included in the analyses. Specifically, the majority of the variables consisted of
fairly crude indicators of either the presence or absence of a particular disease factor and did not
provide a measure of severity. This may have reduced the ability to identify any associations, if
present, between the severity of visible differences from the disease and the presence of
appearance concerns. An additional limitation is that the sample sizes of Black patients and those
who self-identified as members of another racial/ethnic group were small. These limitations may
have been reflected in the model fit statistics, which were less than ideal [28]. It is also possible
that the relatively limited information obtained from the predictive model was due to the
dichotomous outcome variable. Most patients reported the presence of appearance concerns;
however, measuring appearance concerns may not be something easily categorized as present or
absent, and thus, the study results may differ if a more robust measure of appearance concerns
were used. Additionally, the general population sample comparison was done with the only
general population data that were currently available. While it was useful to contextualize
patterns of appearance concerns among patients with SSc, the degree of actual differences may
17
have been influenced by factors not considered in the present analysis. Another limitation is that
the categories for reporting features of concern that were used in the present study were
somewhat broad and non-specific, which did not allow for a detailed description of the features
listed by patients. Nonetheless, they provided a general understanding of the common types of
appearance concerns among patients and underlined that those most important do not vary
substantially across sex or racial/ethnic groups. Finally, we used self-reported race/ethnicity
classifications. Race/ethnicity is a complex construct that may reflect sociocultural aspects of
experience as much as biological aspects. Furthermore, the Other race/ethnicity category allowed
us to separate out people who did not identify as White or Black, but its composition was
heterogeneous and not necessarily informative in relation to the experience of any patient or
group of patients classified in the Other group.
In sum, female SSc patients had significantly higher odds of reporting appearance
concerns than male patients. Although Black SSc patients had higher odds of experiencing
appearance concerns than White patients on an unadjusted basis, this result was not statistically
significant when accounting for the influence of other sociodemographic and disease/medical
characteristics. It may be the case that greater appearance concerns among Black patients reflect
more significant appearance changes, such as skin involvement, including pigmentation changes.
Both women and men with SSc were more likely to report appearance concerns than general
population survey respondents. The difference in reporting of appearance concerns between SSc
patients and the general population was greatest for men and for older patients. In both of these
cases, rates in the general population were relatively low for these groups, but much less so in
SSc. Despite differences in overall rates, across groups defined by sex or race/ethnicity, the most
commonly reported features of appearance concern related to the face and head regions, followed
18
by the hands and fingers. Replications of the current study with larger samples of Black and
other racial/ethnic groups are needed. In addition, future studies should include assessment of
appearance concerns with a continuously measured outcome variable or one that explores
multiple domains of appearance concerns, as well as include factors, such as disease duration and
disease and serological subtype, to explore their potential impact on appearance concerns.
19
ACKNOWLEDGMENTS
This study was supported by funding to the Scleroderma Patient-centered Intervention
Network (SPIN) from the Canadian Institutes of Health Research (PI Thombs; TR3-119192). In
addition to CIHR funding, SPIN has received institutional contributions from the Lady Davis
Institute for Medical Research of the Jewish General Hospital, Montréal, Canada and from
McGill University, Montréal, Canada. SPIN has also received support from the Scleroderma
Society of Ontario, the Scleroderma Society of Canada, and Sclérodermie Québec. Ms. Jewett
was supported by a CIHR Doctoral Research Award. Dr. Kwakkenbos was supported by a CIHR
Banting Postdoctoral Fellowship. Ms. Rice was supported by a FRSQ Masters Training Award.
Dr. Thombs was supported by an Investigator Salary Award from the Arthritis Society. The
authors declare that there are no conflicts of interest to report.
20
REFERENCES
1. Boin F, Wigley FM. Clinical features and treatment of scleroderma. In: Firestein GS, Budd
RC, Gabriel SE, McInnes IB, O’Dell JR, editors. Kelley's textbook of rheumatology. (9th
edition). Philadelphia (PA): Elsevier; 2012. p.1366-1403.
2. Mayes M. Systemic sclerosis: A. clinical features. In: Klippel JH, Stone JH, Crafford LJ,
White PH, editors. Primer on the rheumatic diseases. (13th edition). New York (NY):
Springer and Arthritis Foundation; 2008. p.343-50.
3. Rumsey N, Harcourt D. The psychology of appearance. New York: Open University Press;
2005.
4. Clarke A, Thompson AR, Jenkinson E, Rumsey N, Newell R. CBT for appearance anxiety:
Psychosocial interventions for anxiety due to visible difference. West Sussex: Wiley-
Blackwell; 2013.
5. Ong J, Clarke A, White P, Johnson M, Withey S, Butler PEM. Does severity predict distress?
The relationship between subjective and objective measures of appearance and psychological
adjustment during facial lipoatrophy. Body Image 2007; 4: 239-48.
6. van Lankveld WGJM, Vonk MC, Teunissen H, van den Hoogen FHJ. Appearance self-
esteem in systemic sclerosis – subjective experience of skin deformity and its relationship
with physician-assessed skin involvement, disease status and psychological variables.
Rheumatology. 2007; 46: 872-6.
7. Jewett LR, Hudson M, Malcarne VL, Baron M, Thombs BD, Canadian Scleroderma
Research Group. Sociodemographic and disease correlates of body image distress among
patients with systemic sclerosis. PLOS ONE. 2012; 7: e33281.
21
8. Benrud-Larson LM, Heinberg LJ, Boling C, Reed J, White B, Wigley FM, et al. Body image
dissatisfaction among women with scleroderma: Extent and relationship to psychosocial
function. Health Psychol. 2003; 22: 130-9.
9. Malcarne VL, Hansdottir I, Greenberg HL, Clements PJ, Weisman MH. Appearance self-
esteem in systemic sclerosis. Cognit Ther Res. 1999; 23: 197-208.
10. Kwakkenbos L, Delisle VC, Fox RS, Gholizadeh S, Jewett LR, Levis B, et al. Psychosocial
aspects of scleroderma. Rheum Dis Clin N Am. 2015; 41: 519-28.
11. Sivakumar B, Haloob N, Puri A, Latif A, Ghani V, Brough J, et al. Systemic sclerosis as a
model of chronic rejection in facial composite tissue transplantation. J Plast Reconstr Aesthet
Surg. 2010; 63: 1669-76.
12. Amin K, Clarke A, Sivakumar B, Puri A, Fox Z, Brough J, et al. The psychological impact of
facial changes in scleroderma. Psychol Health Med. 2011; 163: 304-12.
13. Tiggemann M. Body image across the adult life span: Stability and change. Body Image.
2004; 1: 29-41.
14. Harris DL, Carr AT. Prevalence of concern about physical appearance in the general
population. Brit J Plast Surg. 2001; 54: 223-6.
15. Rumsey N, Harcourt D. Body image and disfigurement: Issues and interventions. Body
Image. 2004; 1: 83-97.
16. Hassan J, Grogan S, Clark-Carter D, Richards H, Yates VM. The individual health burden of
acne: Appearance-related distress in male and female adolescents and adults with back, chest
and facial acne. J Health Psychol. 2009; 14: 1105-18.
22
17. Richards HS, Jenkinson E, Rumsey N, White P, Garrott H, Herbert H, et al. The
psychological well-being and appearance concerns of patients presenting with ptosis. Eye
(Lond). 2014; 28: 296-302.
18. Rumsey N. The psychology of facial disfigurement: Implications for whole face
transplantation. Curr Otorhinolaryngol Rep. 2014; 2: 210-6.
19. Thompson A, Kent G. Adjusting to disfigurement: Processes involved in dealing with being
visibly different. Clin Psychol Rev. 2001; 21: 663-82.
20. Rumsey N, Clarke A, White P, Wyn-Williams M, Garlick W. Altered body image:
Appearance-related concerns of people with visible disfigurement. J Adv Nurs. 2004; 48:
443-53.
21. van der Veen JPW. Pigmentary disorders in Western countries. Dermatol Clin. 2007; 25:
449-55.
22. Reveille JD, Fischbach M, McNearney T, Friedman AW, Aguilar MB, Lisse J, et al.
Systemic sclerosis in 3 US ethnic groups: A comparison of clinical sociodemographic,
serologic, and immunogenetic determinants. Semin Arthritis Rheum. 2001; 30: 332-46.
23. Kwakkenbos L, Jewett LR, Baron M, Bartlett SJ, Furst D, Gottesman K, et al. The
Scleroderma Patient-centered Intervention Network (SPIN) Cohort: Protocol for a cohort
multiple randomised controlled trial (cmRCT) design to support trials of psychosocial and
rehabilitation interventions in a rare disease context. BMJ Open. 2013; 3: e003563.
24. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013
Classification criteria for systemic sclerosis: An American College of
Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis
Rheum. 2013; 65: 2737-47.
23
25. Medsger TA Jr, Bombardieri S, Czirjak L, Scorza R, Della Rossa A, Bencivelli W.
Assessment of disease severity and prognosis. Clin Exp Rheumatol. 2003;21:S42-6.
26. Carr T, Moss T, Harris D. The DAS24: A short form of the Derriford Appearance Scale
DAS59 to measure individual responses to living with problems of appearance. Br J Health
Psychol. 2005; 10: 285-98.
27. Moss TP, Lawson V, White P, The Appearance Research Collaboration. Identifying the
underlying factor structure of the Derriford Appearance Scale-24. Peer J. 2015; 3: e1070.
28. Hosmer DW, Lemeshow S. Applied logistic regression. 2nd ed. New York (NY): John Wiley
& Sons, Inc.; 2000.
24
Table 1. Sociodemographic and Disease Characteristics (N = 644)
25
Total Race/Ethnicity Sex
Variable N = 644
White
n = 550
Black
n = 41
Other
n = 53 p value
Female
n = 565
Male
n = 79 p valueAge (years),mean ± SD
55.3 ± 12.1 56.5 ± 11.5a,b 47.7 ± 11.5a 48.1 ± 14.1b <.001 55.1 ± 12.0 56.2 ± 12.8 .462
Education >12 years,n (%)
512 (79.5) 434 (78.9) 33 (80.5) 45 (84.9) .579 451 (79.8) 61 (77.2) .591
Currently employed(full or part-time), n (%)
268 (41.7)c 225 (41.0)d 21 (51.2) 22 (41.5) .439 236 (41.8) 32 (41.0)e .901
Married/living as married, n (%)
471 (73.1) 413 (75.1)a 21 (51.2)a 37 (69.8) .003 412 (72.9) 59 (74.7) .741
Time since onset of first non Raynaud’s symptoms (years), mean ± SD
11.5 ± 8.7f 11.6 ± 8.9g 8.9 ± 5.6h 10.2 ± 7.8i .067 11.7 ± 8.8j 10.4 ± 7.8k .227
Body Mass Index (BMI) mean ± SD
26.04 ± 6.12 26.14 ± 6.14 26.14 ± 6.84 24.97 ± 5.33 .413 26.02 ± 6.26 26.19 ± 5.00 .816
Patients with diffuse SSc, n (%)
258 (40.1) 200 (36.4)a,b 28 (68.3)a 30 (56.6)b <.001 226 (40.0) 32 (40.5) .971
Telangiectasias <.001 .058
None,n (%)
183 (28.4) 137 (24.9)a 26 (63.4)a 20 (37.7) 153 (27.1) 30 (38.0)
Body and face,n (%)
298 (46.3) 273 (49.6)a 7 (17.1)a 18 (34.0) 262 (46.4) 36 (45.6)
Body only,n (%)
163 (25.3) 140 (25.5)a 8 (19.5)a 15 (28.3) 150 (26.5) 13 (16.5)
Pigmentation changes <.001 .497
None,n (%)
438 (68.0) 396 (72.0)a,b 13 (31.7)a 29 (54.7)b 385 (68.1) 53 (67.1)
Body and face, 105 (16.3) 71 (12.9)a,b 18 (43.9)a 16 (30.2)b 89 (15.8) 16 (20.3)
26
Abbreviations: SD = standard deviation.
aWhite patients statistically significantly different from Black patients.bWhite patients statistically significantly different from patients from another racial/ethnic group.cSample size based on N = 643.dSample size based on N = 549.eSample size based on N = 78. fSample size based on N = 595. gSample size based on N = 510. hSample size based on N = 40.iSample size based on N = 45.jSample size based on N = 519.kSample size based on N = 76.
27
Table 2. Appearance Concerns Reported by SSc Patients who Specified Feature of Concern (N = 444)
Total Race/Ethnicity Sex
(n, %)White(n, %)
Black(n,%)
Other(n, %)
Female(n, %)
Male(n, %)
Feature
Body Area-related
Face/mouth/
head/neck
180 (40.5) 149 (40.5) 14 (40.0) 17 (41.5) 159 (41.2) 21 (36.2)
Hands/fingers 119 (26.8) 103 (28.0) 7 (20.0) 9 (22.0) 99 (25.6) 20 (34.5)
Arms 5 (1.1) 3 (0.8) 1 (2.9) 1 (2.4) 5 (1.3) 0
Chest/stomach 9 (2.0) 9 (2.4) 0 0 8 (2.1) 1 (1.7)
Legs/feet 13 (2.9) 12 (3.3) 1 (2.9) 0 12 (3.1) 1 (1.7)
Skin-related
Telangiectasias
(any)
33 (7.4) 28 (7.6) 1 (2.9) 4 (9.8) 26 (6.7) 7 (12.1)
Skin pigmentation
changes (any)
12 (2.7) 9 (2.4) 1 (2.9) 2 (4.9) 10 (2.6) 2 (3.4)
Skin tightening or 7 (1.6) 5 (1.4) 1 (2.9) 1 (2.4) 5 (1.3) 2 (3.4)
28
hardening
Skin
unspecified/other
20 (4.5) 16 (4.3) 1 (2.9) 3 (7.3) 17 (4.4) 3 (5.2)
Weight-related
Loss 4 (0.90) 3 (0.8) 1 (2.9) 0 4 (1.0) 0
Gain/bloating/
swelling
22 (5.0) 17 (4.6) 2 (5.7) 3 (7.3) 22 (5.7) 0
Unspecified 20 (4.5) 14 (3.8) 5 (14.3) 1 (2.4) 19 (4.9) 1 (1.7)
Total 444 (100.0) 368 (100.0) 35 (100.0) 41 (100.0) 386 (100.0) 58 (100.0)
29
Table 3. Unadjusted and Adjusted Comparisons of Appearance Concerns across Sociodemographic and Disease Variables
Variable Unadjusted
OR (95% CI) p value
Adjusted
OR (95% CI)a p value
Race/ethnicity (reference = White)
Black 2.64 (1.01-6.34) .030 1.76 (0.67-4.60) .250
Other 0.92 (0.52-1.65) .788 0.59 (0.31-1.14) .117
Age 0.97 (0.96-0.98) <.001 0.98 (0.96-0.99) .007
Female sex 2.70 (1.73-4.22) <.001 2.97 (1.78-4.95) <.001
Education 12 years 0.69 (0.47-1.02) .063 0.74 (0.48-1.14) .174
Not married/living as married 1.36 (0.92-2.00) .121 1.05 (0.68-1.60) .841
BMI 1.00 (0.92-1.10) .951 1.01 (0.98-1.05) .421
Diffuse disease subtype 1.76 (1.24-2.50) .002 1.18 (0.76-1.83) .456
Telangiectasias (reference = none)
Body and face 0.81 (0.54-1.22) .313 1.48 (0.89-2.47) .136
Body only 0.70 (0.44-1.09) .116 1.20 (0.77-1.86) .420
Pigmentation changes
30
(reference = none)
Body and face 1.58 (0.96-2.58) .071 0.91 (0.52-1.57) .730
Body only 1.50 (0.92-2.47) .108 1.02 (0.51-2.06) .947
Hand contractures (reference =
none/mild)
Moderate 2.33 (1.41-3.86) .001 2.05 (1.17-3.60) .012
Severe 2.03 (0.88-4.70) .099 1.96 (0.75-5.15) .170
Skin thickening on hands 1.64 (1.18-2.28) .003 1.48 (1.00-2.19) .050
Abbreviations: OR = odds ratio; CI = confidence interval; BMI = Body Mass Index.
aAdjusted for age, sex, race/ethnicity, education level, marital status, disease subtype, telangiectasias, pigmentation changes, hand contractures, and skin thickening on fingers.
31
Table 4. Proportion of Women and Men who Reported Appearance Concerns Based on Age in General Population and SPIN Samples
General Population Sample (n, %)a
SPIN Sample(n, %)
Relative RiskSPIN / General
Population (95% CI)Women
Age bands (years)
18-40 367/537 (68) 50/60 (83) 1.22 (1.07-1.38)
41-50 139/226 (62) 80/101 (79) 1.29 (1.12-1.49)
51-60 151/240 (63) 145/183 (79) 1.26 (1.11-1.42)
61+ 58/174 (33) 146/221 (66) 1.98 (1.57-2.50)
Men
Age bands (years)
18-40 152/341 (45) 5/9 (56) 1.25 (0.69-2.26)
41-50 69/170 (41) 8/11 (73) 1.79 (1.20-2.69)
51-60 49/202 (24) 12/22 (55) 2.25 (1.43-3.54)
61 + 45/218 (21) 17/37 (46) 2.23 (1.44-3.44)
aBased on values reported in Harris and Carr, 2001 (14).
32